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Background: In our study, we sought to analyse the mid-term results after interventional aortic coarctation (CoA) stenting with sequential dilation of the stent., Methods: The data of all 218 patients, who are above the age of 6 years and underwent CoA-stent implantation in our hospital, were retrospectively analysed on the rate of re-interventions, complications and arterial hypertension at a follow-up time of 31 months. To avoid any aortic complications, stents were deployed primarily not in full size and a second cardiac catheterisation for further dilatation was scheduled within 6-12 months after the stent implantation., Results: The median peak invasive systolic pressure gradient declined significantly from 26.2 mmHg to 2.7 mmHg after stenting. There was one procedure related death due to an aortic rupture after stent implantation. There were in total 33 (15.1%) procedure-related complications including femoral artery complications, stent fracture and stent dislocation (in 9, 9 and 7 patients, respectively). In 85 patients a re-dilatation and in 25 patients a second stent-implantation was necessary at the first re-intervention. The systolic blood pressure declined significantly from 144 mmHg to 131 mmHg after stenting. The number of patients being normotensive changed from 18% before stenting to 78.5% after stenting with adjusted antihypertensive medication., Conclusion: Aortic stenting is an effective means for CoA treatment. With sequential dilation of the stent, a very low rate of life-threatening procedural complications and mortality can be achieved. CoA stenting with proper antihypertensive medications results in better control of blood pressure., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: We sought to analyze the quality of life and the predictive value of clinical variables from previous follow-up study in patients late after surgical treatment of aortic coarctation on the quality of life., Methods: All patients, who have participated in the prospective cross-sectional COALA Study in 2000 with a structural clinical investigation including blood pressure measurement and symptom-limited exercise test were contacted for the health-related quality of life questionnaire SF-36 from January 2013 through December 2014., Results: From 273 eligible patients, we received data from 135 patients, 9 of them died during the follow-up time at the median age of 46years (range 30-64years). Seventy-four patients did not participate in the study, other 64 patients moved to remote or unknown areas and could not be contacted. Quality of life was good in the fields of physical role and pain. However, patients reported a significant impairment in general health and in health transition, depending on the age. Arterial hypertension and variables from echocardiography or exercise testing from the COALA study were not predictive on functional health status., Conclusion: Quality of life in patients late after aortic coarctation repair is fairly good compared with healthy controls. Impairments in general health and health transition depend mainly on age, can be explained due to numerous comorbidities and reinterventions in long-term. The predictive value of the commonly assessed clinical variables on quality of life is limited., (Copyright © 2017. Published by Elsevier B.V.)

Background: Patients who underwent surgery for aortic coarctation (COA) have an increased risk of arterial hypertension. We aimed at evaluating (1) differences between hypertensive and non-hypertensive patients and (2) the value of cardiopulmonary exercise testing (CPET) to predict the development or progression of hypertension., Methods: Between 1999 and 2010, CPET was performed in 223 COA-patients of whom 122 had resting blood pressures of <140/90 mmHg without medication, and 101 were considered hypertensive. Comparative statistics were performed. Cox regression analysis was used to assess the relation between demographic, clinical and exercise variables and the development/progression of hypertension., Results: At baseline, hypertensive patients were older (p=0.007), were more often male (p=0.004) and had repair at later age (p=0.008) when compared to normotensive patients. After 3.6 ± 1.2 years, 29/120 (25%) normotensive patients developed hypertension. In normotensives, VE/VCO2-slope (p=0.0016) and peak systolic blood pressure (SBP; p=0.049) were significantly related to the development of hypertension during follow-up. Cut-off points related to higher risk for hypertension, based on best sensitivity and specificity, were defined as VE/VCO2-slope ≥ 27 and peak SBP ≥ 220 mmHg. In the hypertensive group, antihypertensive medication was started/extended in 48/101 (48%) patients. Only age was associated with the need to start/extend antihypertensive therapy in this group (p=0.042)., Conclusions: Higher VE/VCO2-slope and higher peak SBP are risk factors for the development of hypertension in adults with COA. Cardiopulmonary exercise testing may guide clinical decision making regarding close blood pressure control and preventive lifestyle recommendations., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Background: Even after excellent repair of aortic coarctation without restenosis there are limitations in exercise capacity at long-term follow-up. This study was performed to assess the contribution of inherited genomic polymorphisms to exercise capacity in patients without restenosis., Patients and Methods: 122 patients aged 17-72 years, 46 female, 76 male, seen 2-27 years after repair of aortic coarctation with a residual brachial-ankle-gradient ≤20 mmHg were investigated. Genomic polymorphism of angiotensin converting enzyme (ACE I/D), angiotensinogen (AGT, c.704C > T), angiotensin II receptor type 1 (AGTR1, c.1166A > C), endothelin 1 (EDN1, EDN1/ex5-c.5665G > T), G protein (GNB3, c.825C > T), and two polymorphisms each of the ß1-adrenoreceptor (ADRB1, c.145G > A and c.1165C > G), ß2-adrenoreceptor (ADRB2, c.46A > G and c.79C > G), and endothelial NO synthase (NOS3, intron 4 I/D and NOS3, c.894G > T) were determined by PCR amplification and fragment length analysis. Exercise capacity was determined by an upright bicycle exercise test., Results: Only the c.46A > G polymorphism of the ADRB2 (p = 0.024) and the c.704T > C AGT polymorphism (p = 0.042) were positively correlated with peak workload. Patients with one or especially two polymorphic alleles showed a significant higher exercise performance compared with those patients homozygous for the wild type., Conclusions: In contrast to a previous study in heart failure patients, after coarctation repair adults had a better exercise capacity with the G allele of the ß2-receptor c.46A > G polymorphism. Therefore, the exercise capacity of coarctation patients does not profit from an enhanced down regulation of their beta receptors.

Objective: Even after repair of aortic coarctation without restenosis there is a high incidence of arterial hypertension. This study was performed to assess the contribution of several inherited gene polymorphisms, which are known to be related to essential hypertension., Patients and Methods: 122 patients aged 17-72 years, 46 women, and 2-27 years after repair of isolated aortic coarctation without restenosis were investigated. Genomic polymorphism of angiotensin converting enzyme (ACE I/D), angiotensinogen (AGT, c.704C>T), angiotensin II receptor type 1 (AGTR1, c.1166A>C), aldosterone synthase (CYP11B2, c.-344C>T), endothelin 1 (EDN1, EDN1/ex5-c.5665G>T), G protein (GNB3, c.825C>T), G protein-coupled receptor kinase 4 (GRK4, c.679C>T), fibrillin 1 (FBN1, VNTR(TAAA)) and two polymorphisms each of the ß1 adrenoreceptor (ADRB1, c.145G>A and c.1165C>G), ß2 adrenoreceptor (ADRB2, c.46A>G and c.79C>G), and endothelial NO synthase (NOS3, intron 4 I/D and NOS3, c.894G>T) were determined by PCR amplification and fragment length analysis. Patients were classified "normotensive", if they were not on antihypertensive drugs and showed normal blood pressure both on ambulatory measurement and exercise test., Results: None of the investigated genomic polymorphism could be related to hypertension. Only patients with the ACE I/I genotype had a less pronounced nocturnal dipping and patients with a ADRB1 c.1165 C/C genotype had a higher systolic and mean blood pressure at night., Conclusions: Development of late hypertension after aortic coarctation repair could not be related to the investigated genomic polymorphism. The correlation of the ACE I/D and the ADRB1 c.1165C>G polymorphism to nocturnal dipping and blood pressure at nighttime needs further confirmation., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Patients after coarctation repair still have an increased risk of cardiovascular or cerebrovascular events. This has been explained by the persisting hypertension and alterations in the peripheral vessels. However, involvement of the central vessels such as the retinal arteries is virtually unknown. A total of 34 patients after coarctation repair (22 men and 12 women; 23 to 58 years old, age range 0 to 32 years at surgical repair) and 34 nonhypertensive controls underwent structural and functional retinal vessel analysis. Using structural analysis, the vessel diameters were measured. Using functional analysis, the endothelium-dependent vessel dilation in response to flicker light stimulation was assessed. In the patients after coarctation repair, the retinal arteriolar diameter was significantly reduced compared to that of the controls (median 182 mum, first to third quartile 171 to 197; vs 197 microm, first to third quartile 193 to 206; p <0.001). These findings were independent of the peripheral blood pressure and age at intervention. No differences were found for venules. The functional analysis findings were not different between the patients and controls (maximum dilation 3.5%, first to third quartile 2.1% to 4.5% vs 3.6%, first to third quartile 2.2% to 4.3%; p = 0.81), indicating preserved autoregulative mechanisms. In conclusion, the retinal artery diameter is reduced in patients after coarctation repair, independent of their current blood pressure level and age at intervention. As a structural marker of chronic vessel damage associated with past, current, or future hypertension, retinal arteriolar narrowing has been linked to stroke incidence. These results indicate an involvement of cerebral microcirculation in aortic coarctation, despite timely repair, and might contribute to explain the increased rate of cerebrovascular events in such patients., (Copyright 2010 Elsevier Inc. All rights reserved.)

Study Design: This is a retrospective cross-sectional study to analyze mortality and the rate of restenosis in the follow-up of patients after surgical repair of isolated aortic coarctation in infancy., Patients and Methods: From 1974 to 2003, 191 patients underwent surgical repair of aortic coarctation in infancy. Follow-up data of 2,432 patient-years were extracted from the clinical files of our outpatient department or from family practitioners., Results: Five patients died (total mortality 2.6%): 1 patient intraoperatively and 2 within 30 days after surgery (early mortality 1.6%). The other 2 patients died within 3 months. All deaths were contributed to patients that underwent surgery in the years up to 1981 and within the first 43 days of life. Of the surviving 186 patients, 31 had reintervention because of restenosis and another 11 patients had a noninvasive brachial-ankle systolic blood pressure gradient >20 mm Hg, suggesting current restenosis. Risk factors for death or restenosis were a hypoplastic aortic arch and a low body length at surgery., Conclusions: Nowadays, surgical repair of coarctation can be performed in infancy with minimal risk. The restenosis rate is considerably high (23%). It is mainly caused by the size of the whole aortic arch, but can also develop during later follow-up., (Copyright 2008 S. Karger AG, Basel.)

There are contradictory reports whether exercise capacity is reduced in patients on long-term follow-up after coarctation repair. Data from unselected patient groups are missing. In a cross-sectional, long-term follow-up study of a tertiary congenital cardiology referral center, 260 patients (30.2+/-11.4 years old, 84 women), after surgical repair for isolated aortic coarctation (age at surgery 11.5+/-11.2 years), underwent a symptom-limited exercise test. Peak workload was 180+/-52 W, significantly less than the age- and height-related reference values (p<0.0005). A peak workload under 80% of expected was found in 200 patients (77%). Exercise performance of the patients was independent from age at surgery, type of surgery, or the systolic brachial-ankle blood pressure difference. The only exercise-limiting factor found was the chronic administration of diuretics to treat hypertension (p=0.005). Exercise hypertension, defined as a systolic blood pressure >2 SD above the load-dependent reference value, was found in 73 patients (28%). It was independently related to the systolic brachial-ankle blood pressure difference (p<0.0005) and diuretics administration (p=0.037). In conclusion, most patients after coarctation repair have a reduced exercise performance. This reduction is not related to the surgical results. Particularly, as these patients are at risk of early atherosclerosis, exercise should be promoted as primary prevention after restenosis, aortic or cerebral aneurysms, and severe exercise hypertension are ruled out.

Objective: Recent studies have demonstrated that there is a loss of aortic compliance in patients after coarctation repair. The clinical effect of this and other mechanisms apart from restenosis on the rate of arterial hypertension is unknown., Methods: From 1974 through 2000, 404 patients born before January 1, 1985, underwent surgical intervention for isolated aortic coarctation. From those 382 who are still alive, 273 patients aged 16 to 73 years (1-27 years after surgical intervention) underwent a structured clinical investigation according to a prospective protocol, including blood pressure measurement at all limbs, ambulatory blood pressure measurement, and symptom-limited exercise testing., Results: Sixty-seven (25%) patients were already taking antihypertensive drugs, and another 63 (23%) patients had an increased ambulatory blood pressure. Still another 26 (10%) patients had a blood pressure during exercise exceeding 2 standard deviations of reference values. Only 117 (43%) patients had a normal blood pressure reaction. From those 156 patients with hypertension, only 21 (13%) had a systolic brachial-ankle blood pressure difference of greater than 20 mm Hg, suggesting restenosis. In the patient group without restenosis (n = 245), independent risk factors for hypertension were repair with prosthetic material, male sex, a residual brachial-ankle blood pressure difference, and older age at follow-up., Conclusions: The majority of patients were hypertensive at long-term follow-up after coarctation repair. This is caused by restenosis, defined by a gradient of greater than 20 mm Hg, in only a few patients. Even in those without prosthetic material or minimal-grade restenosis, there is a substantial incidence of arterial hypertension.

Aims: The current study was designed to assess midterm results of stent implantation into the aorta for native and recurrent coarctation (CoA) in children and young adults., Methods and Results: Forty-three patients (native CoA, 8; female, 12) were treated with stent implantation at a median age of 16.8 years (range 7.9-44.8 years). Only stents dilatable to an adult size aorta were implanted. All but two patients with functionally univentricular hearts had arterial hypertension. Exercise tests, 24-h blood pressure, clinical examination, echocardiography, and elective catheterization were used to assess follow-up. The narrowed segment was widened significantly from a median of 8 to 12.4 mm (P < 0.0005). The peak-to-peak gradient between the ascending and the descending aorta was lowered significantly from a median of 22 mmHg to 1 mmHg (P < 0.0005). No major complications occurred. The systolic blood pressure at the right arm was lowered significantly (P < 0.0005) from 144 mmHg before stent implantation to 128 mmHg at the last visit. At a median follow-up of 30 months (3-72 months), 68% of all patients were classified to be normotensive., Conclusion: Stent implantation for selected patients with recurrent and native CoA is safe and may effectively reduce the blood pressure gradient across the CoA site. We suggest using only stents dilatable to an adult size aorta. However, arterial hypertension persists in a significant number of the patients. Impaired elastic properties of the aorta may be the cause for this finding.

Objectives: To compare images of the aorta obtained with helical CT (HCT) scanning and MRI for the follow-up of adults with coarctation of the aorta (CoA)., Design: Longitudinal study., Setting: Department of adult congenital heart disease in a tertiary university hospital., Patients: A total of 37 adults (age range, 16 to 68 years; women, 13) with CoA (after surgery, 34 patients; native, 2 patients; after balloon-angioplasty, 1 patient), Measurements and Results: All patients underwent both HCT and MRI of the thoracic aorta within a mean (+/- SD) time interval of 1.86 +/- 1.11 years. Aortic diameters measured at six intrathoracic levels showed a high correlation (r = 0.79 to 0.94). On average, slightly lower diameters were measured with MRI (1.2 mm). But there was a substantial variation between the two measurements with differences of up to 9 mm. All other pathomorphologic abnormalities were detected and classified similarly with both methods., Conclusions: HCT and MRI are similarly useful for the noninvasive evaluation of the thoracic aorta in patients with CoA. But there can be a substantial variation in two subsequent measurements without an overall substantial bias toward larger diameter in one of the two methods. In repetitive studies, changes of the diameters should be interpreted with care, especially when assessing the progression of aortic diameters.

Study Design: This is a retrospective cross-sectional study to analyze mortality and the rate of restenosis in the follow-up of patients after surgical repair of isolated aortic coarctation in infancy. Patients and Methods: From 1974 to 2003, 191 patients underwent surgical repair of aortic coarctation in infancy. Follow-up data of 2,432 patient-years were extracted from the clinical files of our outpatient department or from family practitioners. Results: Five patients died (total mortality 2.6%): 1 patient intraoperatively and 2 within 30 days after surgery (early mortality 1.6%). The other 2 patients died within 3 months. All deaths were contributed to patients that underwent surgery in the years up to 1981 and within the first 43 days of life. Of the surviving 186 patients, 31 had reintervention because of restenosis and another 11 patients had a noninvasive brachial-ankle systolic blood pressure gradient >20 mm Hg, suggesting current restenosis. Risk factors for death or restenosis were a hypoplastic aortic arch and a low body length at surgery. Conclusions: Nowadays, surgical repair of coarctation can be performed in infancy with minimal risk. The restenosis rate is considerably high (23%). It is mainly caused by the size of the whole aortic arch, but can also develop during later follow-up. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]